ABSTRACT
BACKGROUND@#The presence of fibrosis is a criterion for subtype classification in the newly updated hypersensitivity pneumonitis (HP) guidelines. The present study aimed to summarize differences in clinical characteristics and prognosis of non-fibrotic hypersensitivity pneumonitis (NFHP) and fibrotic hypersensitivity pneumonitis (FHP) and explore factors associated with the presence of fibrosis.@*METHODS@#In this prospective cohort study, patients diagnosed with HP through a multidisciplinary discussion were enrolled. Collected data included demographic and clinical characteristics, laboratory findings, and radiologic and histopathological features. Logistic regression analyses were performed to explore factors related to the presence of fibrosis.@*RESULTS@#A total of 202 patients with HP were enrolled, including 87 (43.1%) NFHP patients and 115 (56.9%) FHP patients. Patients with FHP were older and more frequently presented with dyspnea, crackles, and digital clubbing than patients with NFHP. Serum levels of carcinoembryonic antigen, carbohydrate antigen 125, carbohydrate antigen 153, gastrin-releasing peptide precursor, squamous cell carcinoma antigen, and antigen cytokeratin 21-1, and count of bronchoalveolar lavage (BAL) eosinophils were higher in the FHP group than in the NFHP group. BAL lymphocytosis was present in both groups, but less pronounced in the FHP group. Multivariable regression analyses revealed that older age, <20% of lymphocyte in BAL, and ≥1.75% of eosinophil in BAL were risk factors for the development of FHP. Twelve patients developed adverse outcomes, with a median survival time of 12.5 months, all of whom had FHP.@*CONCLUSIONS@#Older age, <20% of lymphocyte in BAL, and ≥1.75% of eosinophil in BAL were risk factors associated with the development of FHP. Prognosis of patients with NFHP was better than that of patients with FHP. These results may provide insights into the mechanisms of fibrosis in HP.
Subject(s)
Humans , Bronchoalveolar Lavage Fluid , Prospective Studies , Alveolitis, Extrinsic Allergic/diagnosis , Fibrosis , CarbohydratesABSTRACT
Background: Ursodeoxycholic acid (UDCA) is currently used for the treatment of primary biliary cholangitis (PBC), but some people do not respond well to UDCA. It reported that the combination of fenofibrate and UDCA can improve the clinical indices in these patients. However, more high-quality evidence is needed to improve guideline recommendations. Methods: Through an updated meta-analysis, studies included were valued by the Cochrane Evaluation Manual and Robins-I. Biochemical and clinical indicator changes in UDCA-refractory PBC patients receiving combination therapy were analyzed by Revman 5.42. Then, we explored the influence of fenofibrate dose and the effectiveness and safety of long-term application by retrospective cohort study. Results: Our meta-analysis included nine publications with a total of 389 patients, including 216 treated with UDCA alone and 173 who received combination therapy. The meta-analysis showed that combination therapy was more effective than UDCA monotherapy in decreasing biochemical parameters, such as ALP, GGT, IgM, and TG. However, the occurrence of pruritus and adverse events was slightly higher with combination therapy than with UDCA monotherapy. A total of 156 patients were included in our cohort study: 68 patients underwent UDCA monotherapy, and 88 patients underwent combination therapy. Among UDCA-refractory patients, fenofibrate add-on therapy significantly improved the ALP normalization rate. Conclusion: The combination of fenofibrate and UDCA can decrease biochemical parameters, of UDCA-refractory PBC patient. Furthermore, the efficacy and safety of long-term combination therapy were also confirmed in our cohort study.
ABSTRACT
Primary biliary cholangitis (PBC) is an autoimmune liver disease manifesting as cholestasis and is often observed in the middle-aged and elderly women. About 50% of the patients have fatigue and itching, and 20% have depression or mood changes. In recent years, a number of studies have shown that the non-specific symptoms of patients with primary biliary cholangitis (PBC), such as fatigue, itching, and cognitive changes, are associated with the structural and functional changes of the central nervous system. Early identification of preclinical PBC patients through brain imaging changes may be one of the ways for the early diagnosis of this disease.
ABSTRACT
Primary biliary cholangitis (PBC) is an autoimmune liver disease manifesting as cholestasis and is often observed in the middle-aged and elderly women. About 50% of the patients have fatigue and itching, and 20% have depression or mood changes. In recent years, a number of studies have shown that the non-specific symptoms of patients with primary biliary cholangitis (PBC), such as fatigue, itching, and cognitive changes, are associated with the structural and functional changes of the central nervous system. Early identification of preclinical PBC patients through brain imaging changes may be one of the ways for the early diagnosis of this disease.
